DISEASE SCANNER

Global Incurable Diseases Tracker

Back to Globe
Genetic Disorder

Congenital Diaphragmatic Hernia (CDH)

HIGH SEVERITY

A birth defect where the diaphragm fails to form completely, allowing abdominal organs to move into the chest and compress lungs. Causes pulmonary hypoplasia and pulmonary hypertension. Life-threatening condition requiring immediate intervention.

Global Affected

35.0K

Countries

15

Symptoms

Respiratory distress at birth
Scaphoid abdomen
Barrel chest
Cyanosis
Heart displacement
Pulmonary hypertension
Persistent fetal circulation

Treatment Options

Genetic counseling
Supportive care
Symptom management
Enzyme replacement (if applicable)
Physical therapy
Regular monitoring
Multidisciplinary care

Risk Factors

1Family history
2Genetic mutations
3Consanguinity
4Advanced paternal age
5Ethnic predisposition

Diagnostic Methods

  • 1Genetic testing
  • 2Clinical evaluation
  • 3Family history assessment
  • 4Specialized laboratory tests
  • 5Imaging studies
  • 6Biopsy (if applicable)

Prognosis

Variable by severity. Survival 70-90% overall. Severe cases: 50% survival. Long-term complications: pulmonary hypertension, gastroesophageal reflux, hearing loss. Most survivors have good quality of life.

Prevention

  • Genetic counseling
  • Carrier screening
  • Prenatal diagnosis
  • Preimplantation genetic diagnosis
  • Family planning

Research Status

Fetal intervention (tracheal occlusion) for severe cases. Immediate resuscitation and ventilation. ECMO support. Surgical repair when stable. Survival 70-80% overall; <50% for severe cases. Long-term respiratory and GI follow-up.

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK1116
  • https://medlineplus.gov/genetics
  • https://rarediseases.org

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.